Monday, May 2, 2011

Adenocarcinoma rare Cancers Of The Bladder

Glandular metaplasia is common in the urothelium, and the appearance of cystitis cystica and glandular cystitis are known consequences of chronic infection, inflammation or irritation (as urolithiasis). In the transformed state, focal areas of glandular differentiation are reasonably common in invasive non-papillary TCC. To our knowledge, focal areas of glandular differentiation have no clinical significance with regard to natural history or response to commonly used systemic agents. Moreover, it is rare to see a remnant of glandular differentiation in a piece of cystectomy after neoadjuvant chemotherapy for high grade, papillary TCC, even when nothing is seen adenocarcinomatous before therapy. These relatively common clinical scenarios highlight the morphological repertoire urothelial histology, and illustrate the difficulty of defining precisely what is meant by "adenocarcinoma of the bladder." In this section we limit our discussion to cancers that have adenocarcinoma histology as the dominant model, and recognizing the CTP.

There are many variants of adenocarcinoma found in the bladder. Most authors (including the World Health classification of tumors of the bladder) include mucinous ring, seal, type enteric hepatoid, and clear cell (formerly mesonephric) as recognizable subsets. In addition, an adenoid cystic pattern can also be seen, especially in the context of the transformation of a preexisting cystic cystitis. Tumors that mixtures of these patterns are the rule. Enteric type histology is particularly found among the cancers that originate in a urachal remnant, and these are taken separately in the "urachal cancer" below. However, villous adenoma and intestinal-type adenocarcinoma do occur rarely in the bladder properly. The immunophenotype of these cancers tend to overlap with that observed in colon cancer, and most produce carcinoembryonic antigen (CEA).

Most of the adenocarcinomas arising in the bladder are of the variety suitable mucinous or seal ring. The male / female ratio is at least 2: 1, and age of onset is very similar to that observed in conventional CTP. Bladder exstrophy, a rare developmental anomaly affecting 1 in 50 000 births, is a well-established risk factor, like other non-physiological states as a urinary bladder out of place. Intestinal metaplasia long before the appearance of carcinoma is typical in these contexts. Adenocarcinomas also arise in the context of preexisting cystic cystitis (and glandular), sometimes in association with schistosomiasis (although squamous histology is more common in the context of the latter).

It is typical to be diffusely infiltrative mucinous adenocarcinomas, and present with irritative symptoms out of proportion with the results of cystoscopy. Cross-sectional imaging typically shows diffuse thickening of the bladder walls, and plastic linitis Frank is well known, particularly in the subgroup with predominant signet ring histology. We have encountered patients not only with plastic linitis, but also with the involvement of seminal vesicles, and the extension, even to the spermatic cords. Thus, while the appearance may be mediocre cystoscopy, examination under general anesthesia, is striking.

The clear cell variant of adenocarcinoma of the bladder is very rare, and is also clinically distinct. These cancers often occur in women (at least 2: A female predominance) and the median age is younger. More than half of the cases appear to arise from the urethra or periurethral glands. Usually express CA125, and there are other lines of evidence supporting an etiology of M ullerian rest.

These cancers tend to be very sensitive to taxane-based therapy, such as those used for epithelial ovarian cancer. The optimal clinical management of adenocarcinoma is, of course, are not established. The stadium is the most important prognostic factor, and unfortunately the majority of patients with adenocarcinoma presented with locally advanced disease (cT3 or higher).

Therefore, for many patients, neoadjuvant systemic therapy is a reasonable consideration as historical results for surgery alone in this environment is poor. Most patients in our center are, in fact, treated with combination chemotherapy followed by radical surgery. We have seen the answers to a variety of chemotherapy regimens.

In the metastatic setting, we find many examples of patients who had an excellent response to chemotherapy, including the standard therapy for TCC. However, the overall response rate is lower than seen with conventional TCC, and the survival of more than 2 years is uncommon